Choledochal cysts (CC) are rare congenital, cystic dilations of the biliary tree occurring predominantly in Asian populations and females. Patients usually children presenting with any following: abdominal pain, palpable mass, jaundice. Its congenital nature hints at a potential genetic cause. A possible causal gene is TP53, tumor suppressor germline variant called rs201753350 (c.91G>A) that changed from G allele to an allele, decreasing cell proliferation suppressing activity its functional...

We report two infants with neonatal cholestasis and hepatosplenomegaly secondary to congenital syphilis. The onset of jaundice the first infant was at six weeks life second case on 28th hour associated neurologic bone involvement. diagnosis suspected based a maternal history untreated syphilis, clinical findings, reactive rapid plasma reagin. Early recognition treatment can lead improvement but prevention by mandatory testing syphilis is more effective strategy

Jaundice in older children can occur when any obstructon is found within the bile ducts, either from stones,parasites, choledochal cysts and rarely, secondary to tumors. We present a previously well, 10-year-old Filipino boy with three-week history of progressive jaundice tea-colored urine, was initally assessed have biliary ascariasis. Ultrasound showed heterogeneous focus spanning gallbladder neck confrmed on endoscopic retrograde cholangiopancreatography as an exophytc mass at ampulla...

Background: Acute cholangitis (AC) in children is a rare but life-threatening infection. Symptoms vary from mild to severe disease. There are no local published data on pediatric AC. Objective: To determine the clinical, biochemical, ultrasonographic, microbiologic features, and treatment outcome of patients with definite Methodology: Cross-sectional study using medical records diagnosed AC based Modified Tokyo Guidelines 2018 admitted January 2016 June 2021. Results: Twenty-seven aged 0 18...

Background. Extrahepatic biliary atresia (EHBA) causes a rare obstructive cholestasis in infants. Kasai portoenterostomy if done before the third month of life may relieve obstruction. Genetic predisposition has been implicated EHBA etiopathogenesis with rs17095355 polymorphism having strongest association. We determined association between and susceptibility Filipino children, described outcome each genotype among timely operated patients. Methods. Thirty-four histologically diagnosed...

Objective. To determine if the CD14/-159 and TNFα/-308 single nucleotide polymorphisms (SNPs) are associated with development of Idiopathic Neonatal Hepatitis (INH) in Filipino children. Methods. Genomic DNA from 33 patients diagnosed INH age- sex-matched controls, children without any liver disease, were recruited. Baseline serum total bilirubin (TB), direct (DB), alkaline phosphatase (ALP) obtained their medical records. Genotypes for CD14/159 determined via PCR sequencing. Results. No...

Objective. To describe the clinical, biochemical, microbiologic, radiologic and histological features outcome of intestinal TB.Methods. Medical records patients diagnosed with TB were reviewed. Cases considered bacteriologically-confirmed if tissue was positive on smear culture or polymerase chain reaction (PCR); clinically-diagnosed histologic, evidence extra-pulmonary TB. Results. Fifteen [Mean (SD) age: 13 (4) years; 53% females] included. One bacteriologicallyconfirmed; fourteen...

Background. Human Pegivirus (HPgV), previously called Hepatitis G virus or GB C, is an RNA virus. It can be transmitted vertically (mother to infant), parenterally and sexually. HPgV share common routes of transmission other viruses such as B virus, C Immunodeficiency (HIV) thus co-infection usually observed. Risk groups include injection drug users, HIV-positive individuals, multi-transfused patients, hemodialysis hemophiliacs, chronic liver disease patients organ transplant recipients. The...

Background. The features and outcome of hepatobiliary tuberculosis (HBTB) have not been extensively reportedin children.
 Objective. To describe the clinical, biochemical, radiologic, microbiologic histologic ofchildren diagnosed with HBTB.
 Methods. Data HBTB patients aged 0-18 years were collected by review medical records as they wereadmitted. Cases classified bacteriologically-confirmed (positive AFB smear, TB culture or PCR bile/livertissue) clinically-diagnosed (clinical,...

A 12-year-old female had a three-year history of fever, non-bilious vomiting and abdominal pain. Upper gastrointestinal series showed filling defect at the duodenum. Esophagogastroduodenoscopy exhibited circumferentialmass extending from duodenal bulb to 2nd part duodenum which on histology disclosed chronicgranulomatous inflammation. Chest X-ray suggested miliary tuberculosis; endotracheal tube aspirate was PCRpositive for Mycobacterium tuberculosis. Patient diagnosed as disseminated...

We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity ofintrahepatic bile ducts and triangular facies. Eight associated congenital heart disease (six pulmonicstenosis, one each tetralogy Fallot patent ductus arteriosus), seven with butterfly vertebrae onewith posterior embryotoxon. Seven the are alive jaundice-free but three concomitanthypercholesterolemia; six other died liver-related complications.

KEY FINDINGS
 There is conflicting evidence on the efficacy of intravenous immunoglobin G in treatment COVID-19 
 patients with severe disease. Intravenous immunoglobulin (IVIg) a mixture polyclonal (IgG3, IgG4) antibodies as well variable amounts proteins; IgA, IgE and IgM isolated pooled from healthy donors. IgG involved viral neutralization, modulates anti-inflammatory cytokines cytokine antagonists.
 Immediate adverse effects IVIg include flu-like syndrome, dermatologic...

Objective. To determine factors predictive of obstructive neonatal cholestasis among Filipino infants and to describe their outcome.
 Methods. Jaundiced within the first eight weeks life with liver biopsy were included. Excluded secondary metabolic or infective causes. Retrospective chart review (2009-2012) prospective recruitment patients (2013) done. A final diagnosis non-obstructive was made on clinical, biochemical, ultrasonographic, histologic findings, using histology and/or...

Objective. We investigated the effect of personal protective equipment (PPE) on prevention COVID-19 health care workers (HCW) assigned in wards, intensive (ICU) and emergency room (ER). Methods. searched MEDLINE, Cochrane CENTRAL as 30 April 2021, well trial registers, preprint sites living evidence sites. included studies that compared use PPE versus no COVID-19. screened studies, extracted data, assessed risk bias certainty using GRADE approach. Results. Five observational (three cohort...

Background. Vitamin D deficiency occurs in 10% to 36% of children with cholestasis. The relationship between serum vitamin levels, severity liver disease and bone abnormalities has not been extensively investigated. Objective. To determine levels its association presence radiographic rickets Methodology. Children aged 0–10 years cholestasis underwent 25-hydroxyvitamin (25-[OH]D) determination, radiographs wrists knees function tests. Liver was evaluated using the Child-Pugh score....

Systemic Lupus Erythematosus (SLE) is a multi-systemic autoimmune disorder. Fifty percent will have gastro intestinal symptoms and 1 to 4% jaundice. Two Filipino adolescents with uncommon presentation of SLE are reported: 14-year old female intermittent epigastric pain 17-year male Epigastric jaundice presentations in patients SLE. A high index suspicion needed must be considered the presence other clinical immunologic features.